scholarly journals Subpial Aspirated Cerebellar Tonsils in Pediatric Chiari I Malformation: anatomopathological study

2019 ◽  
Vol 29 (3) ◽  
pp. 501-505
Author(s):  
Bruna Lisboa do Vale ◽  
Maurus Marques de Almeida Holanda ◽  
Diogo Berto Campos ◽  
Moisés Dantas Cartaxo de Abreu Pereira ◽  
Érico Samuel Gomes Galvão da Trindade

Introduction: Chiari Malformation (CM) is a condition in which ectopy or herniation of components of the posterior cranial fossa to theforamen magnum are present, and can be divided into four types. Type I (CM-I) occurs when there is protrusion of only the cerebellartonsils into the cervical spinal canal. One of the available therapeutic options is subpial aspiration. Previous anatomopathologicalstudies showed that the main alterations in the aspirated tonsils were the loss of Purkinje cells, Bergmann gliosis, cortical atrophyand neuronal changes due to hypoxia; all of them secondary to the aggression to the nervous tissue over time. These changes leadto gradual loss of function of the tissue, with compensation by other structures. Therefore, when subpial aspiration of the herniatedtonsil is performed in adults, there are no negative consequences to the patient in the future, as concluded by many studies targetingadults with CM. However, studies in children with CM are scarce, which means that we cannot affirm in which age the histologicalchanges occur. Hence, we cannot determine whether or not the surgical removal of this tissue in childhood leads to future losses tothe patient. Objective: To analyze the main histological aspects of cerebellar tonsils in children with Chiari I Malformation whounderwent subpial aspiration, to help clarify the natural history of this pathology, as well as to help determine the prognosis of thispopulation when treated with this technique. Method: We analyzed the histopathology of the tonsils removed with subpial aspirationtechnique, in several hospitals of João Pessoa, Brazil, from children diagnosed with Chiari I malformation. Results: The slides of alltonsils showed the same histological changes: Bergmann gliosis, loss of Purkinje cells, atrophy of the cerebellar cortex and meningealfibrosis. The obtained results were similar to the ones found in previous studies carried out in adults. Conclusion: The cerebellaranatomopathological alterations secondary to Chiari I Malformation, which leads to loss of tissue function, seem to appear early inlife, suggesting the safety of the subpial aspiration in pediatric patients. However, more extensive studies and long-term follow-up ofpatients are needed to establish with more precision the natural history of the disease.

2021 ◽  
Vol 14 (6) ◽  
pp. e241789
Author(s):  
Hadleigh Cuthbert ◽  
Joshua Pepper ◽  
Rupert Price

The Chiari I malformation (CM-I) is characterised by overcrowding of the posterior fossa and descent of the cerebellar tonsils and is associated with syringomyelia. With the increasing availability of magnetic resonance imaging, CM-I is placing a growing burden on neurosurgical services. However, its natural history remains poorly understood, and the timing and nature of surgical intervention is controversial. We present a case of a significant, symptomatic CM-I with associated syrinx which underwent complete spontaneous resolution over a 4-year period. Spontaneous regression of Chiari malformation and syringomyelia is exceedingly rare; a literature review reveals 15 other cases and only one case which underwent complete resolution. The present case and literature review suggest a more benign natural history of CM-I and support a more conservative approach to its management. Further studies are required to determine whether any factors can predict resolution for certain patient cohorts.


Author(s):  
Matthew Carey ◽  
William Fuell ◽  
Thomas Harkey ◽  
Gregory W. Albert

1993 ◽  
Vol 107 (5) ◽  
pp. 441-443 ◽  
Author(s):  
F. W. J. Albers ◽  
K. J. A. O. Ingels

The type I Chiari malformation consists of a caudal displacement of the cerebellar tonsils through the foramen magnum into the cervical spinal canal. The most common presenting symptoms, such as pain, weakness and headache, are frequently preceded by otoneurological symptoms. Sensorineural hearing loss, vertigo, nystagmus, dysequilibrium, tinnitus and other cranial nerve involvement have been reported in Chiari-I malformation. A case report is presented and the clinical features of the disease are discussed with emphasis on the otoneurological aspects.


2006 ◽  
Vol 194 (5) ◽  
pp. 552-560 ◽  
Author(s):  
Elizabeth Margaret Maloney ◽  
Yoshihisa Yamano ◽  
Paul C. VanVeldhuisen ◽  
Takashi Sawada ◽  
Norma Kim ◽  
...  

Neurosurgery ◽  
2012 ◽  
Vol 71 (3) ◽  
pp. 594-603 ◽  
Author(s):  
Bradley A. Gross ◽  
Rose Du

Abstract BACKGROUND: Hemorrhage from cerebral dural arteriovenous fistulae (dAVF) is a considerable source of neurological morbidity and even mortality. OBJECTIVE: To evaluate the natural history of cerebral dAVF. METHODS: We reviewed our own cohort of 70 dAVF and incorporated results from the literature, synthesizing pooled hemorrhage rates and evaluating risk factors for 395 dAVF in 6 studies. RESULTS: No hemorrhages occurred during 409 lesion-years of follow-up of Borden type I dAVF; however, cortical venous drainage developed in 1.4%. Like type I dAVF, type II dAVF demonstrated a female predilection and were most commonly transverse-sigmoid or cavernous. Eighteen percent of type II dAVF presented with hemorrhage (95% confidence interval [CI]: 8%-36%), and the annual hemorrhage rate was 6% (95% CI: 0.1%-19%). Borden type III dAVF demonstrated a male predilection and were most commonly tentorial or petrosal. Thirty-four percent presented with hemorrhage (95% CI: 0.4%-49%), with an annual hemorrhage rate of 10% (95% CI: 4%-20%), increasing to 21% for those with venous ectasia (95% CI: 4%-66%). The hemorrhage rate decreased to 2% for asymptomatic or minimally symptomatic type II or III dAVF (95% CI: 0.2%-8%), and increased to 10% for those presenting with nonhemorrhagic neurological deficits (95% CI: 0.9%-41%) and to 46% for those presenting with hemorrhage (95% CI: 11%-130%). CONCLUSION: Venous ectasia is a significant risk factor for hemorrhage among dAVF with cortical venous drainage. In addition, those with hemorrhagic presentation, even compared with nonhemorrhagic neurological deficit presentation, as well as Borden type III dAVF compared with type II dAVF demonstrated a trend toward greater hemorrhage rates.


Neurosurgery ◽  
2005 ◽  
Vol 57 (2) ◽  
pp. E371-E371 ◽  
Author(s):  
Mustafa Efkan Colpan ◽  
Zeki Sekerci

ABSTRACT OBJECTIVE AND IMPORTANCE: We report on a patient with a Chiari I malformation presenting with right hemifacial spasm. Clinicians should consider the downward displacement of the hindbrain as a rare cause of hemifacial spasm in Chiari I malformation. CLINICAL PRESENTATION: An 18-year-old man was admitted with right hemifacial spasm. The results of the neurological examination were normal except for the facial spasm. Magnetic resonance imaging demonstrated a Chiari I malformation without syringomyelia. After surgery, the hemifacial spasm completely resolved. INTERVENTION: Posterior fossa decompression, C1 laminectomy, and duraplasty were performed. CONCLUSION: The hemifacial spasm could be attributed to compression and/or traction of the facial nerve because of downward displacement of the hindbrain in Chiari I malformation. Compression and/or traction might create irritation of the facial nerve that causes hemifacial spasm. Resolution of the hemifacial spasm after posterior fossa decompression could explain the facial nerve irritation in Chiari I malformation. Clinicians should consider Chiari malformation as a cause of hemifacial spasm and posterior fossa decompression as a potential treatment.


2010 ◽  
Vol 76 (6) ◽  
pp. 599-605 ◽  
Author(s):  
Joshua D. Adams ◽  
Margaret C. Tracci ◽  
Sahir Sabri ◽  
Kenneth J. Cherry ◽  
John F. Angle ◽  
...  

Endoleaks are a frequent complication of thoracic endovascular aortic repair (TEVAR) and will likely increase in incidence with application of the technique to more complicated aortic anatomy and a wider range of thoracic aortic pathologies. Management generally consists of aggressive repair of Type I endoleaks; however, the natural history of Type I endoleaks after TEVAR remains largely unknown. The purpose of this study was to examine the incidence and characteristics of Type I endoleaks and to evaluate clinical outcomes of patients with Type I endoleaks after TEVAR. A single-center retrospective review was performed on all patients who underwent TEVAR over a 4-year period. Type I endoleaks were detected in 21 per cent (27 of 129) of patients on post-deployment aortography or CT angiography. During a mean follow-up of 750.63 ± 483 days, 59 per cent (16 of 27) closed spontaneously; 30 per cent (eight of 27) required secondary endovascular intervention; and 11 per cent (three of 27) have persisted with no increase in maximum aortic diameter. No patients have died or required open surgical conversion as a result of their Type I endoleak. Although accurate predictors of spontaneous resolution of Type I endoleaks have yet to be definitively characterized, our initial results suggest that it may be safe to observe small Type I endoleaks given that a large percentage resolve spontaneously and no endoleak-related deaths have occurred.


2019 ◽  
Vol 10 ◽  
pp. 78
Author(s):  
Aslam Hentati ◽  
Mohamed Badri ◽  
Kamel Bahri ◽  
Ihsen Zammel

Background: The Type I malformations are supposed to be the result of mesodermal defects that create a congenitally small posterior fossa. However, Chiari malformation could be also “iatrogenic” and then called “acquired” Chiari I malformation. In this study, the authors report the clinical feature of a patient who developed a Chiari I malformation after lumboperitoneal shunt. Case Description: A 35-year-old woman has been suffering from idiopathic intracranial hypertension and rhinorrhea due to an anterior skull base defect. A valveless lumboperitoneal shunt followed by surgical closure of the defect was performed. Six months later, she suffered from major continuous occipital headaches. The neurological examination found a mild cerebellar gait ataxia and cerebellar dysarthria. The cerebral magnetic resonance imaging (MRI) showed a ptosis of the cerebellar tonsils and a disappearance of the cisterna magna; there was no syringomyelia. This herniation was not present before shunt was performed. A replacement of the lumboperitoneal shunt with a pressure-regulated valve chamber was performed. After a 1-year follow-up, the patient reports a marked decrease of the headache as well as the ataxia, and the last cerebral MRI showed resolution of the Chiari I malformation. Conclusions: Symptomatic acquired Chiari malformation with or without syringomyelia as a delayed complication after lumbar shunting is a rare complication, particularly reported in the pediatric population, but could also occur to adult patients. Treating these patients by correcting the shunt’s valve could be enough, but should be monitored, as it may fail to resolve the Chiari malformation even years after treatment.


2019 ◽  
pp. 57-64
Author(s):  
Yiping Li ◽  
Bermans J. Iskandar

Chiari I malformation (CIM) is defined by pathological herniation of the cerebellar tonsils below the foramen magnum. Operative intervention for CIM is generally undertaken to treat neurological deficits associated with tonsillar herniation or with associated syringomyelia. A complete brain and spine MRI is indicated to rule out associated abnormalities and to identify and the presence and extent of syringomyelia. The type of surgical decompression remains controversial but may include bone-only decompression, bony decompression followed by duraplasty, and bony decompression followed by duraplasty and tonsillar shrinkage. Post-operative monitoring for CSF leakage, inadequate decompression, subtle chronic craniocervical instability, and hydrocephalus is critical.


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